Masataka Kuwana1, Yuichiro Shirai2, Tsutomu Takeuchi2. 1. From the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.M. Kuwana, MD, PhD, Professor, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; Y. Shirai, MD, PhD, Instructor, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; T. Takeuchi, MD, PhD, Professor, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine. kuwanam@nms.ac.jp. 2. From the Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.M. Kuwana, MD, PhD, Professor, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; Y. Shirai, MD, PhD, Instructor, Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine; T. Takeuchi, MD, PhD, Professor, Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine.
Abstract
OBJECTIVE: To identify predictors of poor prognosis in patients with systemic sclerosis (SSc) associated with interstitial lung disease (ILD). METHODS: Fifty patients with early-stage SSc-ILD who had never received disease-modifying drugs and were either observed for ≥ 10 years or died from ILD-related causes were enrolled. The baseline variables of patients who developed endstage lung disease (ESLD) were compared with those of patients who remained ESLD-free, and the Cox proportional hazard model was used to identify initial factors that correlated with ESLD development. RESULTS: Sixteen patients (32%) developed ESLD during 173.5 ± 64.7 months of followup. Elevated serum Krebs von den Lungen-6 (KL-6) at initial assessment was highly correlated with ESLD development (p = 0.0002). Receiver-operating characteristic curve analysis revealed that a KL-6 value of 1273 U/ml effectively discriminated patients who developed ESLD from those who did not. Patients with KL-6 > 1273 U/ml were less likely to remain ESLD-free compared with those with lower KL-6 levels (p < 0.0001). Multivariate analysis showed that KL-6 > 1273 U/ml was the most reliable predictor of ESLD development (OR 51.2, 95% CI 7.6-343, p < 0.0001). Finally, the initial KL-6 level correlated with the forced vital capacity (FVC) decline rate (r = 0.58, p < 0.0001). CONCLUSION: The natural course of SSc-ILD is highly variable. Baseline serum KL-6 is a biomarker potentially useful for predicting FVC decline.
OBJECTIVE: To identify predictors of poor prognosis in patients with systemic sclerosis (SSc) associated with interstitial lung disease (ILD). METHODS: Fifty patients with early-stage SSc-ILD who had never received disease-modifying drugs and were either observed for ≥ 10 years or died from ILD-related causes were enrolled. The baseline variables of patients who developed endstage lung disease (ESLD) were compared with those of patients who remained ESLD-free, and the Cox proportional hazard model was used to identify initial factors that correlated with ESLD development. RESULTS: Sixteen patients (32%) developed ESLD during 173.5 ± 64.7 months of followup. Elevated serum Krebs von den Lungen-6 (KL-6) at initial assessment was highly correlated with ESLD development (p = 0.0002). Receiver-operating characteristic curve analysis revealed that a KL-6 value of 1273 U/ml effectively discriminated patients who developed ESLD from those who did not. Patients with KL-6 > 1273 U/ml were less likely to remain ESLD-free compared with those with lower KL-6 levels (p < 0.0001). Multivariate analysis showed that KL-6 > 1273 U/ml was the most reliable predictor of ESLD development (OR 51.2, 95% CI 7.6-343, p < 0.0001). Finally, the initial KL-6 level correlated with the forced vital capacity (FVC) decline rate (r = 0.58, p < 0.0001). CONCLUSION: The natural course of SSc-ILD is highly variable. Baseline serum KL-6 is a biomarker potentially useful for predicting FVC decline.
Authors: Elizabeth R Volkmann; Donald P Tashkin; Masataka Kuwana; Ning Li; Michael D Roth; Julio Charles; Faye N Hant; Galina S Bogatkevich; Tanjina Akter; Grace Kim; Jonathan Goldin; Dinesh Khanna; Philip J Clements; Daniel E Furst; Robert M Elashoff; Richard M Silver; Shervin Assassi Journal: Arthritis Rheumatol Date: 2019-11-01 Impact factor: 10.995